Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature, characterized by relentless deterioration and death. Patients with PAH are known to be at increased risk for anesthetic complications and surgical morbidity and mortality. However, outcomes in patients have improved with the recent development of new drug therapies. The 3 major drug classes for treatment of PAH are prostanoids, endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors. In this review, the authors provide an overview of each drug class, its mechanism of action, indications, and current supportive literature. Surgical and interventional treatments of PAH, including atrial septostomy, pulmonary thromboendarterectomy, and transplantation, are briefly reviewed, and the rationale, indications, and selection criteria for each are discussed. Although available medical and surgical therapies for PAH have improved patient outcomes, acute decompensated right heart failure (RHF) remains a common and challenging complication of PAH. The authors review this topic and provide an outline of the general pathophysiology of RHF and an approach to its management.